Macrophage activation syndrome in the era of biologic therapy

AA Grom, AC Horne, F De Benedetti - Nature Reviews Rheumatology, 2016 - nature.com
Nature Reviews Rheumatology, 2016nature.com
Macrophage activation syndrome (MAS) refers to acute overwhelming inflammation caused
by a'cytokine storm'. Although increasingly recognized as a life-threatening complication of
various rheumatic diseases, clinically, MAS is strikingly similar to primary and secondary
forms of haemophagocytic lymphohistiocytosis (HLH). Not surprisingly, many
rheumatologists prefer the term secondary HLH rather than MAS to describe this condition,
and efforts to change the nomenclature are in progress. The pathophysiology of MAS …
Abstract
Macrophage activation syndrome (MAS) refers to acute overwhelming inflammation caused by a'cytokine storm'. Although increasingly recognized as a life-threatening complication of various rheumatic diseases, clinically, MAS is strikingly similar to primary and secondary forms of haemophagocytic lymphohistiocytosis (HLH). Not surprisingly, many rheumatologists prefer the term secondary HLH rather than MAS to describe this condition, and efforts to change the nomenclature are in progress. The pathophysiology of MAS remains elusive, but observations in animal models, as well as data on the effects of new anticytokine therapies on rates and clinical presentations of MAS in patients with systemic juvenile idiopathic arthritis (sJIA), provide clues to the understanding of this perplexing clinical phenomenon. In this Review, we explore the latest available evidence and discuss potential diagnostic challenges in the era of increasing use of biologic therapies.
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